Aydinok Yesim
Department of Pediatric Hematology and Oncology, Ege University Children's Hospital, Bornova, Izmir 35100, Turkey.
Hematol Oncol Clin North Am. 2018 Apr;32(2):261-275. doi: 10.1016/j.hoc.2017.12.002. Epub 2018 Jan 17.
Introduction of MRI techniques for identifying and monitoring tissue iron overload and the current understanding of iron homeostasis in transfusion-dependent (TDT) and non-transfusion-dependent thalassemia have allowed for a more robust administration of iron chelation therapies. The development of safe and efficient oral iron chelators and the insights gained from large-scale prospective studies using these agents have improved iron overload management. A significant reduction in iron toxicity-induced morbidity and mortality and improvements in quality of life were observed in TDT. The appropriate management of tissue-specific iron loading in TDT has been portrayed using evidence-based data obtained from investigational studies.
用于识别和监测组织铁过载的MRI技术的引入,以及目前对输血依赖型(TDT)和非输血依赖型地中海贫血中铁稳态的认识,使得铁螯合疗法的应用更加完善。安全有效的口服铁螯合剂的开发以及使用这些药物的大规模前瞻性研究获得的见解,改善了铁过载的管理。在TDT中观察到铁毒性引起的发病率和死亡率显著降低,生活质量得到改善。TDT中组织特异性铁负荷的适当管理已通过从研究性研究中获得的循证数据进行了描述。